Cholesterol deficit but not accumulation of aberrant sterols is the major cause of the teratogenic activity in the Smith-Lemli-Opitz syndrome animal model

Low cholesterol and high 7-dehydrocholesterol (7DHC) levels are associated with a blockade of δ7-reductase in the Smith-Lemli-Opitz syndrome (SLOS) and in the animals treated with the inhibitor AY9944. The impact of the cholesterol deficit and of the accumulation of 7DHC on the embryo were investiga...

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Bibliographic Details
Main Authors: Wassila Gaoua, Claude Wolf, Françoise Chevy, Françoise Ilien, Charles Roux
Format: Article
Language:English
Published: Elsevier 2000-04-01
Series:Journal of Lipid Research
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520324123